Presentation of Superior Vena Cava Syndrome as a Consequence of Fibrosing Mediastinitis

Fibrosing mediastinitis (FM) is a rare inflammatory reaction within the mediastinum that causes diffuse fibrosis and can lead to compression of vascular structures in the thoracic and cervical regions. While typically benign and indolent, significant progression can lead to obstruction of vital organs, causing conditions like superior vena cava (SVC) syndrome, pulmonary hypertension, and potentially fatal right-sided heart failure. The clinical presentation often mimics malignancy, which necessitates exclusion. FM is a rare condition, with idiopathic cases being even rarer. While histoplasmosis or tuberculosis infections are common causes of FM in the US, idiopathic forms such as IgG4-related FM can also occur.

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Publication Details

Journal
Cureus
Published
2026-01-08
DOI
https://doi.org/10.7759/cureus.101085
Primary Topic
IgG4-Related and Inflammatory Diseases
Type
article
Field-Weighted Citation Impact
0.00
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article

Presentation of Superior Vena Cava Syndrome as a Consequence of Fibrosing Mediastinitis

Amelia Dorr, Kaitlyn M Unterman, Kathleen Clark, Christopher Gamard
Cureus
IgG4-Related and Inflammatory Diseases
article

Presentation of Superior Vena Cava Syndrome as a Consequence of Fibrosing Mediastinitis

Amelia Dorr, Kaitlyn M Unterman, Kathleen Clark, Christopher Gamard
article en

Abstract

Fibrosing mediastinitis (FM) is a rare inflammatory reaction within the mediastinum that causes diffuse fibrosis and can lead to compression of vascular structures in the thoracic and cervical regions. While typically benign and indolent, significant progression can lead to obstruction of vital organs, causing conditions like superior vena cava (SVC) syndrome, pulmonary hypertension, and potentially fatal right-sided heart failure. The clinical presentation often mimics malignancy, which necessitates exclusion. FM is a rare condition, with idiopathic cases being even rarer. While histoplasmosis or tuberculosis infections are common causes of FM in the US, idiopathic forms such as IgG4-related FM can also occur.

CureusVol. 18(1)
Alabama College of Osteopathic Medicine (US), Mobile Infirmary (US)
Reduced inequalities
Openalex Percentile: Top 94%
IgG4-Related and Inflammatory Diseases
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